Juvenile Nasopharyngeal Angiofibroma

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1 : Juvenile Nasopharyngeal Angiofibroma By Ashwin H Aslam AR
2 : Juvenile angiofibroma (JNA) is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males. Its benign but locally invasive and destroys the adjoining structures Wednesday, May 18, 2011 2 INTRODUCTION
3 : Exact cause – Unknown Adolescent males – predominant Testosterone dependent Hamartamatous nidus of vascular tissue in nasopharynx Aetiology Testosterone Angiofibroma
4 : Arise from post. part of nasal cavity close to the sup. margin of sphenopalatine foramen Site of origin
5 : Angiofibroma made up of vascular and fibrous tissue Vessels are just endothelium lined spaces with no muscle coat Severe bleeding as vessels lose ability to contract, also bleeding cant be controlled by application of adrenaline Pathology
6 : Nasal cavity – nasal obstruction,epistaxis,nasal discharge Paranasal sinuses Pterygomaxillary, infratemporal fossa, cheek Orbits – Proptosis and “frog face deformity”. Enters through inf. or sup. orbital fissure Cranial cavity – middle cranial fossa (common) Enters through floor of middle cranial fossa Through sphenoid sinus Extensions
7 : Profuse & recurrent epistaxis – anaemic Progressive nasal obstruction & denasal speech (due to mass in postnasal space) Conductive hearing loss & serous otitis media (due to obstruction of eustachian tube) Mass in nasopharynx Sessile, lobulated or smooth tumour Pink or purplish Firm in consistency ( digital palpation should never be done) Clinical Features
8 : Broadening of nasal bridge Proptosis Swelling of cheek, infratemporal fossa Involvt. of II, III, IV, VI cranial nerves …clinical features
9 : Getting tensed ??? Don’t worry yaar…. Management is coming…
10 : Soft tissue lateral film of nasopharynx shows soft tissue mass in nasopharynx X – ray CT scan MRI Carotid angiography Investigations
11 : X-ray of paranasal sinuses and base of skull Displacement of nasal septum Opacification of sinuses Ant. bowing of post. wall of maxillary sinus Destruction of medial antral wall Erosion of greater wing of sphenoid or pterygoid plates Widening of lower lateral margin of sup. orbital fissure X- ray
12 : CT scan of head with contrast enhancement Extent of tumour Bony destruction or displacements Antral sign / Holman-Miller sign ( pathognomic ) Anterior bowing of posterior wall of maxillary sinus CT Scan
13 : When soft tissue extensions present intracranially, in infratemporal fossa or into orbit MRI
14 : Shows Extension of tumour Vascularity Feeding vessels ( branches of external carotid system) Here Angiography confirms the hypervascularity of the lesion, which is supplied by a hypertrophic maxillary artery (arrow). Carotid angiography
15 : Based on clinical picture Biopsy of tumour- bleeding If needed, done under gen. anaesthesia Diagnosis
16 : Other causes of nasal obstruction, nasal polyps, antrochoanal polyp, teratoma, encephalocele, dermoids, inverting papilloma, rhabdomyosarcoma, squamous cell carcinoma Other causes of epistaxis, systemic or local Other causes of proptosis or orbital swellings   Wednesday, May 18, 2011 16 Differentials
17 : Surgery Radiotherapy Hormonal Chemotherapy Treatment
18 : Depending on origin & extensions various approaches Transpalatine (tumours confined to nasopharynx) Transpalatine + Translabial (Sardana’s approach) Extended lateral rhinotomy (for tumour & its extensions) Via facial incision Via degloving approach Extended Denker’s approach Intracranial-extracranial Infratemporal fossa Endoscopic Transmaxillary (Le Fort I approach) Maxillary swing approach Surgery
19 : About 2 lit. blood loss Reduce vascularity preoperatively Oestrogen therapy ( stilboestrol 2.5 mg tid 3wks) reduce vascularity Also cryotherapy of tumour, embolisation of feeding vessels reduce vascularity ….
20 : 3000-3500 cGy in 15-18 fractions in 3-3.5 wks Tumour regresses slowly in about a year Radiotherapy
21 : As the primary or adjunctive treatment since tumour occurs in young males at puberty Diethylstilboestrol and flutamide used Hormonal
22 : Recurrent and residual lesions Doxorubicin, vincristine and dacarbazine in combination Chemotherapy
23 : Excessive bleeding can occur. Malignant transformation. Transient blindness as a result of embolization, but it is a rare occurrence. Osteoradionecrosis and/or blindness due to optic nerve damage may occur due to radiotherapy. Fistula of the palate at the junction of the soft and hard palate may occur with the transpalatal approach but is prevented by preservation of the greater palatine vessels during flap elevation. Anesthesia of the cheek is a frequent occurrence with the Weber-Ferguson incision. Wednesday, May 18, 2011 23 Complications
24 : Hippocrates described the tumor in the 5th century BC Friedberg first used the term angiofibroma in 1940 JNA occurs exclusively in males. Females diagnosed with JNA should undergo genetic testing. Do u know !!!
25 : JNA accounts for 0.05 % of all head and neck tumors. A frequency of 1:5,000 - 1:60,000 in otolaryngology patients has been reported. Wednesday, May 18, 2011 25 Epidemiology…!
26 : Thank You…


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